14 cases were investigated between 1963 to 1988. Age ranging from 25-66 (mean 48.2). 9 cases (64%) were over age 50. Diagnosis was established by renal biopsy or autopsy. 11 cases (79%) were primary. 3 cases (21%) were secondary with chronic infection. Besides renal involvement, primary amyloidosis principally affected the heart, gastrointestinal tract and tongue. Hepatosplenomegaly and anemia were found in secondary cases. Proteinuria was found in 14 cases. Nephrotic Syndrome (NS) found in 8 cases, both renal insufficiency and hypertension were 21% respectively. In 3 cases performed renal venography, 2 cases showed renal vein thrombosis. 9 cases (64%) showed kidneys were enlarged. Congo red test showed positive reaction were 100% in secondary and 50% in primary. By light microscopy stained with congo red there was a specific protein deposited in glomeruli and mesangium, by electron microscopy. There was fine amyloid fibrils. These findings are characteristic and to have diagnostic value.
Conclusion: (1) Renal amyloidosis is not very rare in China. (2) Proteinuria and NS were major clinical manifestations. (3) Renal biopsy and autopsy have diagnostic value, congo red test does not prove the absence of primary amyloidosis.